recurrent acute pancreatitis in bowel malrotation...in literature, only two cases were reported with...

Abstract. – OBJECTIVE: Recurrent acutepancreatitis is an uncommon diagnosis inteenagers. Excluded alcohol and biliary stones,more prevalent aetiologies in these group ofpatients are genetic pancreatitis, pancreaticduct system abnormalities, neoplasia, traumasand congenital abnormalities of the duodenumsuch as duodenal duplication or diverticulum.Two reported cases of recurrent pancreatitisassociated to midgut malrotation were de-scribed in English literature. Bowel malrotationis a difficult diagnosis in a teenager or a youngadult and a common delay is documented.Ladd’s procedure is largely recognized to bethe standard treatment for a symptomatic mal-rotation.

CASE REPORT: Our Report describes in de-tails a case of recurrent pancreatitis, where alate diagnosis of midgut malrotation was ob-tained and an endoscopic management was at-tempted. A literature review and an analysis oftwo previously reported cases were performedto explore a possible aetiopathogenesis of therecurrent acute pancreatitis in patients withmidgut malrotation.

RESULTS: 24 months of follow-up showed anasymptomatic patient on a free diet, with a mildderanged Liver Functional Tests and a normalAmylase and Lipase. The recurrence of acutepancreatitis has not been observed until thepresent day.

CONCLUSIONS: Recurrent episodes of acutepancreatitis in young adults, without a historyof alcohol abuse or evidence of gallstones,might be an atypical presentation of midgutmalrotation and it should be in the differentialdiagnosis. In this case, a Ladd’s operation isbeneficial and an endoscopic procedure doesnot obtain advantages.

Key Words: Recurrent acute pancreatitis, Uncommon diagnosis in

young adult, Bowel malrotation, Ladd’s operation.

European Review for Medical and Pharmacological Sciences

Recurrent acute pancreatitis in bowel malrotation

G. ALESSANDRI, A. AMODIO1, L. LANDONI1, N. DE’ LIGUORI CARINO, C. BASSI1

Corresponding Author: Giorgio Alessandri, MD; e-mail: [email protected] 4719

Introduction

Midgut malrotation is a not so rare congenitalabnormality; occurring in one in 500 live birth1.The majority (approximately 80%)1,2 of thesechildren develop gastrointestinal (GI) symptomsin the first month of their life. Midgut malrota-tion is described as a variation of the normal270° counter clockwise rotation that starts afterthe fifth week of pregnancy. Generally, sympto-matic patients present with an obstructive clinicalpicture; vomiting (generally bilious content), in-termittent abdominal pain, diarrhoea, constipa-tion, malabsorption and failure to thrive3. Midgutmalrotation is always associated with an in-creased risk of bowel volvulus and a related is-chemic gut.Pancreatitis is an atypical presentation of an

abnormal intrauterine development of gastro-en-teric apparatus. Pancreatitis during childhood isgenerally observed in children with genetic orcongenital diseases causing cystic dilatation ofbile ducts, maljunction of biliopancreatic ducts,pancreas divisum or other HPB malformation4-7.Causes of pancreatitis in an older age group (upto 25-30) are alcohol, CBD stones, trauma andcongenital abnormalities of the duodenum suchas duodenum duplication, intra or extra luminaldiverticula. Rarely pancreatitis associated withmidgut malrotation were documented in the liter-ature; only two patients with recurrent episode ofacute pancreatitis and a diagnosis of bowel mal-rotation were reported8-11.

Case Report A 24-years-old woman was referred to our

specialist Hepato-Biliary-Pancreatic (HPB) Cen-tre for recurrent episodes of acute pancreatitis of

2016; 20: 4719-4724

HPB Unit, Department of Surgery, Manchester Royal Infirmary Hospital, Central Manchester Foundation Trust, Manchester, UK1Surgical Department, Pancreas Centre, Hospital of “G.B. Rossi”, University of Verona, Verona, Italy

G. Alessandri, A. Amodio, L. Landoni, N. de’ Liguori Carino, C. Bassi

the peritoneum, a remnant of dorsal mesogastri-um. The duodenum was twisted and mild retract-ed upward, the small bowel was situated to theright side of abdomen and the colon was on theleft. The CBD, the main pancreatic duct (MPD)and the duodenum were anterior to the usualanatomy, more floppy and with an unnatural mo-bility. The pancreaticobiliary junction was de-scribed as normal. Neither of the CBD and theMPD were dilated, nor filling defects were ap-preciated and a normal pancreatic outflow wasobserved after the secretin stimulation. More in-vestigations were requested and an endoscopicretrograde cholangiopancreatography (ERCP)showed: “a prepapillary stricture of the mainpancreatic duct without upstream dilatation”. Apancreatic sphincterotomy was performed during

unknown aetiology. Symptoms initially appearedin a fit and well patient 3 years previously; theywere characterized by an epigastric and right up-per quadrant colicky abdominal pain, very severe(VAS 10/10), radiated to the right back, with agradual onset, associated with nausea and severalepisode of vomiting. On examination, the ab-domen was tender in all upper quadrants withguarding on the right side but no rebound was es-timated. First hospital assessment was in January2009, blood showed mild deranged amylase (111UI/L) and Lipase (160 UI/L) with normal LiverFunctional Tests (LFTs). Three ultrasound scans(USS) could not show abnormalities. A CT scan(Figure 1) described an uncomplicated oedema-tous pancreatitis of body and tail of the gland andsludge within the gallbladder. The Common bileduct (CBD) was reported not delated, the duode-num and the head of the pancreas were not de-scribed. A diagnosis of biliary pancreatitis wasmade and an elective laparoscopic cholecystecto-my was performed after a full recovery from theepisode of acute pancreatitis. The procedure wasuneventful. Before the operation, a magnetic res-onance cholangiopancreatography (MRCP) wasobtained: “there were no gallstones, not dilata-tion of the CBD nor pancreatic duct” and therewas not a further description of upper gastroin-testinal (UGI) tract.During the following year, the patient’s symp-

toms relapsed twice with a similar presentation;hospital admission and management with simpleanalgesia were required. Eighteen months post-surgery recurrent

episode of acute pancreatitis lead to a further ad-mission. Investigations showed four times elevat-ed amylase and lipase. Several abdominal and apelvic ultrasounds were requested consecutively,no abnormalities were reported. A new MRCPestimated normal biliary tree and pancreatic duct,a CT scan with contrast showed no bowel ob-struction but a “dysmorphic head of the pancreasand a folded, festooned duodenum” were report-ed. An oesophago-gastro-duodenal endoscopy(OGD) was performed. It described “a consider-able amount of biliary reflux into the stomachand normal first and second portion of duode-num”. An MRCP with secretin (Figure 2) wasscheduled in a middle-high volume HPB centreto review the function of the Oddi sphincter. Dur-ing this procedure, a bowel malrotation was de-scribed. The duodenum and the head of the pan-creas were not lying on a retroperitoneal space,both of them were located between two layers of

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Figure 1. The first CT scan performed on the patientshowed the inverted relationship between superior mesen-teric artery and vein, that was not reported.

Figure 2. MRCP with secretin revealed the uncommon pre-sentation of the head of pancreas and the MPD (arrows). TheCBD and MPD were not dilated, nor filling defects were ap-preciated and a normal pancreatic outflow were observed.

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the ERCP and a plastic stent (4 cm, 5 Fr) left insitu for few days. A functional stricture and a bentduodenum were also confirmed. After that procedure, the symptoms did not dis-

appear and several episodes of recurrent pancre-atitis were observed after that. Nine months laterthere was a new hospital admission. Amylase ele-vated at 6000 UI/L with mild deranged LFTs.USS and MRCP confirmed the uncomplicatedpancreatitis and the ERCP showed a patentWirsung and no stricture on the papilla. The pa-tient was referred to our centre for recurrent pan-creatitis.Here a new assessment was obtained and all

performed investigation reviewed. No mutationof CFTR and SPINK-1 were found on genetictests. A contrast follows through (Figure 3)showed the bowel presentation. It was advocatedto be responsible for a functional, intermittentobstruction of the duodenum. Thus, the increas-ing pressure inside the bowel leads to recurrenceof symptoms. The mobility of the head of thepancreas was considered responsible for thefunctional obstruction to the pancreatic outlet. ALadd’s procedure was scheduled and performedfor the patient. A contrast follow through (Figure4) was obtained one month after the procedure.No recurrence of symptoms has been observedafter 24 months of follow-up.

Discussion

In literature, only two cases were reportedwith an association between midgut malrotationand recurrent episodes of acute pancreatitis8-11.The first case described8 is a retrospective hy-

pothesis that remains without objective proof.

The diagnosis of incomplete bowel rotation,Ladd ligament and an abnormal presentation ofthe duodenum were done only many years afterthe episodes of acute pancreatitis. At 9 and 10years old the child had had two admissions witha diagnosis of idiopathic acute pancreatitis.Cholelithiasis, biochemical and infective aetiolo-gy had been excluded. Several USS had beenperformed at that time but a diagnosis of malro-tation had not been obtained. The patient, at theage of 17, underwent to an exploration laparoto-my for an acute intestinal obstruction. During theprocedure, it was found a volvulus of the rightcolon secondary to a midgut malrotation. Au-thors just mentioned the attractive hypothesis ofa recurrent acute pancreatitis due to a “distortionof the ampulla”, secondary to an uncommon pre-sentation of the duodenum.The second case11 is a 16 years old girl with a

6 years history of worsening recurrent acutepancreatitis. Several investigations were under-taken. A CT scan did not show abnormalities ofthe biliary three, pancreas divisum or annularpancreas. A follow through study showed thepresence of bowel malrotation and barium ene-ma confirmed it. An ERCP was performed andit reported “a distortion of the first and secondpart of the duodenum associated with a mobilehead of the pancreas. The CBD was mild dilat-ed but the pancreatic duct was unremarkableand there was no abnormality of the pancreatic-biliary junction”. A laparoscopic Ladd’s proce-dure was performed eventually. There was norecurrence of symptoms during the 3 years fol-lowing the procedure.

Figure 3. A preoperative contrast follow through showingthe bowel presentation.

Figure 4. A contrast follow through obtained one monthafter Ladd’s procedure. Arrows point at the duodenopexyperformed.

Sasaki et al11 probed a possible aetiology withseveral investigation. Symptoms were related toan unusual feature of the duodenum and pancre-atic head, twisted and retracted upward. This un-usual UGI presentation was assumed to be re-sponsible for a functional, intermittent pancreaticduct obstruction and consequently recurrent pan-creatitis. Findings obtained in that patient by afollow through study and an ERCP supported thehypothesis. The lack of recurrence of symptomsduring the following 3 years of follow-up sup-ported authors opinion.It is well documented as an inflammation of the

pancreas can be obtained in an experimental wayon mice, blocking the proximal part of the smallbowel, distally to the papilla of Vater and before it,increasing the intraduodenal local pressure12.The non-specific presentation of our patient

causes difficulty in establishing a diagnosis. Afterthe first episode of abdominal pain, she was man-aged as a biliary pancreatitis13. A conservativemanagement of pancreatitis and an elective la-paroscopic cholecystectomy were performed14,15.Recurrence of symptoms were carefully investi-gated by MRCP, CT abdomen, USS and a MRCPwith secretin (Figure 2). Eventually, an ERCPwas scheduled. It reported: “a prepapillary stric-ture of the main pancreatic duct without up-stream dilatation and normal outflow, in a patientwith bowel malrotation”. According to this diag-nosis, a pancreatic sphincterotomy was per-formed, but recurrences were observed again.When the patient arrived in our centre, a simpleUGI series (Figure 3) was requested and the casediscussed in a multi-disciplinary meeting. Allavailable imagines were reassessed. A midgutmalrotation with a Ladd’s ligament was con-firmed and judged to be responsible for recurrentpancreatitis. Intraoperative findings supportedour impression. In patient assessed in our centre,there were no signs of biliary obstruction. Therewas no abnormality in the main pancreatic duct,that was no dilated and without any irregularityof the wall. The unusual presentation of the duo-denum and the small bowel were remarkable.The duodenojejunal junction was not found inthe conventional position. The duodenum had anirregular twisted course, located in the right up-per quadrant of the abdomen and with a Z shape(Figure 3). Peritoneal bands entrapped the de-scending and transverse portion of the duodenum(Ladd’s bands), responsible for its fixity to theretroperitoneal space, showing a variable degreeof bowel obstruction. The pancreatic head and

the latter part of the duodenum were anterior tothe retroperitoneal plan, with a degree of mobili-ty. This increased the possibility of an obstruc-tion of the MPD as reported by Sasaki et al11.The duodenum was closely related to a malrotatedcecum and ascending colon. They were attachedto the right side of the abdomen by peritonealbands (Ladd’s ligament) including part of the duo-denum. In this patient a pancreatic sphincterotomyand a stent did not settle the symptoms, showingthat pancreatic duct obstruction was not related toa “distortion of the Ampulla of Vater”, as men-tioned by Kirby et al8. The particular presentationof the proximal midgut instead might be advocat-ed as responsible of a variable duodenal obstruc-tion. This type of bowel obstruction, with an inter-mittent kinking of the pancreatic duct, might beconsidered in our patient as a possible contribu-tive aetiopathogenic mechanism of the recurrentacute pancreatitis.In all these cases there was a delay in the diag-

nosis of midgut malrotation. Spigland et al10 re-ported generally the delay of 20 months to reachthe diagnosis of malrotation in teenager. In ourcase, symptoms initially started in January 2009and the abnormal bowel presentation was detect-ed in September 2010. Numerous USS, MRI andCT scans were performed.Reassessing all available imaging of our pa-

tient obtained before the diagnosis, it was possi-ble to observe typical features of incomplete ro-tation of gut. US, CT and MRI are not the pre-ferred modalities to make a diagnosis of malrota-tion. Careful evaluation of all bowel and the in-verted relationship between superior mesentericartery and vein16-18 (Figure 1) can help to under-stand the anatomy. The gold start investigation toassess the first part of the GI tract is the contrastfollow through study. There are three main radio-logical signs to detect an UGI malrotation: (1)abnormal position of the duodenojejunal junc-tion; (2) spiral, “corkscrew” or Z-shaped courseof duodenum and proximal jejunum; (3) a je-junum located in the right quadrants. In some pa-tients, a contrast enema might be useful to con-firm the diagnosis, as it can show the large bowellocated on the left side of the abdomen.In our case and in the case described by Sasaki

et al, the Ladd’s operation was performed elective-ly, which is widely recognized as the procedure ofchoice to correct a bowel malrotation. Several au-thors advocate a surgical correction in all patientwith a diagnosis of malformation19,20, regardlessthe presence of symptoms. A significant percent-


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age of patients dies or suffers substantial morbidi-ty due to loss of gut, secondary to a complicatingvolvulus and ischemic gut1-21. Asymptomatic pa-tients with a bowel malrotation will always havean increased risk of volvulus22-23.Our patient underwent an endoscopic pancre-

atic sphincterotomy before surgery. Recurrenceof symptoms followed in several occasions. Inour institute, an open Ladd’s procedure was per-formed. During the laparotomy there was a con-firmation of the diagnosis. Ladd’s band was in-cised, the duodenum was carefully mobilized andmanipulated straight. A duodenopexy was creat-ed and the duodenum was relocated to the rightside of the abdomen in a non-twisted position, asdescribed by Bax and Van der Zee24. The appen-dectomy was not conducted and broadening ofthe mesentery was deemed not necessary25,26. Thesurgical procedure was based on intraoperativefinding; a “complete not rotation” pattern27, thececum located on the right lower quadrant and ageneral intra-abdominal assessment suggested abroad mesentery with a low risk for complicatingvolvulus after duodenopexy22,23.

Conclusions

Congenital abnormalities have to be consid-ered as a possible etiology of recurrent acutepancreatitis in a teenager, when the abuse of al-cohol and the diagnosis of gallstones are exclud-ed. The gold standard to make a diagnosis ofmidgut malrotation remain the gastrografin fol-low through study. However, a careful assess-ment of the presentation of the small bowel andthe relationship between the mesenteric vesselscan be used in all other cross section studies toobtain the diagnosis. A symptomatic patient withmidgut malrotation needs a Ladd’s procedure.Recurrent acute pancreatitis can be included inthe clinical picture of a symptomatic patient withmidgut malrotation.

–––––––––––––––––-––––Conflict of InterestThe Authors declare that they have no conflict of interests.

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recurrent acute pancreatitis in bowel malrotation...in literature, only two cases were reported with...

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